Structured Summary
Abstract
A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
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Broader headings
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MeSH Record
Synonyms
21 entry terms
- 2-Oxoisocaproate Dehydrogenase
- 2-Oxoisovalerate Dehydrogenase (Lipoamide)
- Alpha-Keto Acid Dehydrogenase
- BCKA Decarboxylase
- Branched Chain Alpha-Keto Acid Decarboxylase
- Branched Chain Ketoacid Dehydrogenase
- Branched-Chain 2-Oxo Acid Dehydrogenase
- Branched-Chain Keto Acid Dehydrogenase
- Branched-Chain Oxo-Acid Dehydrogenase
- 2 Oxoisocaproate Dehydrogenase
- Acid Dehydrogenase, Alpha-Keto
- Alpha Keto Acid Dehydrogenase
- Branched Chain 2 Oxo Acid Dehydrogenase
- Branched Chain Alpha Keto Acid Decarboxylase
- Branched Chain Keto Acid Dehydrogenase
- Branched Chain Oxo Acid Dehydrogenase
- Decarboxylase, BCKA
- Dehydrogenase, 2-Oxoisocaproate
- Dehydrogenase, Alpha-Keto Acid
- Dehydrogenase, Branched-Chain Oxo-Acid
- Oxo-Acid Dehydrogenase, Branched-Chain
MeSH Record
Aspects Covered
29 allowable subheadings
Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, deficiency, drug effects, economics, genetics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, physiology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, ultrastructure, urine.
MeSH Record
History Note
2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
MeSH Record
Previous Indexing
- Amino Acids, Branched Chain (1979-1981)
- Carboxy-Lyases (1974-1979)
- Keto Acids (1973-1981)
- Ketone Oxidoreductases (1982-2003)
- Multienzyme Complexes (1982-2003)
MeSH Hierarchy
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AMA Style
References
- National Library of Medicine. 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide). Medical Subject Headings (MeSH). 2026. Unique ID D042942. http://id.nlm.nih.gov/mesh/2026/D042942
- 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide). In: Wikipedia. https://en.wikipedia.org/wiki/Branched-chain_alpha-keto_acid_dehydrogenase_complex
- 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide). In: Wikidata. https://www.wikidata.org/wiki/Q22327132